Full text article. Also available at https://doi.org/10.1093/omcr/omaa024Sex cord tumour with annular tubules (SCTAT) is uncommon and distinctive type of sex cord-stromal tumours of the ovary which develops from sex cord cells. Most of SCTATs are strongly associated with Peutz–Jeghers syndrome (PJS) and have low malignancy potential; however, 20% of non-PJS-associated SCTATs have been reported to have high malignancy potential. Herein, we present a 13-year-old female who presented with severe abdominal pain localized in the right lower side, associated with nausea. Based on histopathological and immunohistochemical findings, the diagnosis was confirmed to be SCTAT. SCTAT of the ovary is extremely rare in the paediatric population as compared to the general population. Its occurrence among paediatrics as it was the case in the patient described in this paper may pose diagnostic challenges due to lack of clinical suspicion and therefore resulting in delay of diagnosis.